The purpose of this study is to estimate the sensibility at the growth hormone in vivo at the children presenting a Prader-Willi syndrome (SPW) in comparison with children presenting a deficit in growth hormone (GHD).

drug : the treatment will be begun in progressive dose by beginning by ¼ of the dose the first week, then ½ of the dose the second week, then 3/4 of the dose the third week and total dose the fourth week.

drug : the treatment will be begun in progressive dose by beginning by ¼ of the dose the first week, then ½ of the dose the second week, then 3/4 of the dose the third week and total dose the fourth week.

drug : the treatment will be begun in progressive dose by beginning by ¼ of the dose the first week, then ½ of the dose the second week, then 3/4 of the dose the third week and total dose the fourth week.

Patient with Prader-Willi Syndrome taking growth Hormone and who has biopsy

Procedure: biopsy

Biopsy : Cutaneous and fat tissue biopsy.

Detailed Description:

Estimate the sensibility at the growth hormone in vivo at the children presenting a Prader-Willi syndrome (SPW) in comparison with children presenting a deficit in growth hormone (GHD) by the measure of the circulating rates of IGF-I under treatment.

Eligibility

Ages Eligible for Study:

1 Year to 5 Years

Genders Eligible for Study:

Both

Accepts Healthy Volunteers:

No

Criteria

Inclusion Criteria:

SPW and SPW-B :

Female or male child of age > or = 1 year

Child naïve of treatment by GH and that must begin a treatment with GH

Child covered by a national insurance scheme or an equivalent

Signature of the informed consent by one of both holders of the parental authority

GHD :

Female or male child of age > or = 1 year

Child paired for the age (+/-on 1 year) and for the sex with regard to the group SWP

Child naïve of treatment by GH and that must begin a treatment with GH

Child covered by a national insurance scheme or an equivalent

Signature of the informed consent by one of both holders of the parental authority * The deficit in GH can be isolated or associated with one or several other hormonal deficits: deficit in TSH, deficit in ACTH, deficit in LH-FSH, deficit in prolactin. The child GHD can thus receive other treatments associated with the growth hormone.

T : controls

Female or male child of age > or = 1 year

Child paired for the age (+/-on 1 year) and for the sex with regard to the group SWP

Child hospitalized at the hospital of the children of the University Hospital of Toulouse for a programmed surgical operation

Child covered by a national insurance scheme or an equivalent

Signature of the informed consent by one of both holders of the parental authority

SPW-GH-B :

Female or male child of age > or = 1 year

Child hospitalized for a programmed surgical operation

Child covered by a national insurance scheme or an equivalent

Child treated with GH for at least 3 month

Signature of the informed consent by one of both holders of the parental authority

Exclusion Criteria:

SPW and GHD

Child presenting a contraindication to the taking of growth hormone :

Growth cartilage welded

Tumoral pathology in process of evolution

Corticosteroid therapy (not substitute)

Allergy known about solvent

Badly balanced diabetes

Child presenting a hypersensitivity to the active principle or to one of the excipients of Genotonorm ® or Omnitrope ®

Child presenting a hypersensitivity to the local anaesthetic with amide connecion

Child presenting a hypersensitivity to the components of the bandage Emlapatch®

Child presenting a hypersensitivity to one of the components of the lidocaïne aguettant without conservative®

Child presenting a porphyria

Child presenting a congenital methemoglobinemia

Child presenting a contraindication to Meopa : patients requiring a ventilation in pure oxygen, intracranial High blood pressure, Any change of the state of consciousness, preventing the cooperation of the patient, Pneumothorax, Bubbles of emphysema, Gaseous embolism, Accident of dive, abdominal gaseous Distension, Patient having received recently an ophthalmic gas (SF6, C3F8, C2F6) used in the eye surgery as long as persists a bubble of gas inside the eye and at least during a period of 3 months. Grave postoperative complications can arise in touch with the increase of the pressure intraocular, facial Traumatism interesting the region of application of the mask

T : controls

Chronicle pathology in which an abnormality of growth would be involved

Other hormonal abnormalities

Children receiving a treatment on the long range, corticosteroid therapy in particular, being able to interfere with the sensibility to GH or to the insulin

Holder of the parental authority under supervision, guardianship or under protection of justice

Participation in another study simultaneously at this one

Contacts and Locations

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Please refer to this study by its ClinicalTrials.gov identifier: NCT01298180